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J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.43.5.458 on 1 May 1980. Downloaded from

Journal of Neurology, Neurosurgery, and Psychiatry, 1980, 43, 458-460

Short report Trigeminal sensory neuropathy and bilateral syndrome: the initial manifestation of mixed connective tissue disease

F M VINCENT AND R N VAN HOUZEN From the Munson Medical Center, Traverse City, Michigan, USA

SUMMARY A patient is described who developed a bilateral carpal tunnel syndrome and a tri- geminal sensory neuropathy as the initial manifestations of mixed connective tissue disease. Either condition can occur in other connective tissue diseases, but this combination has not been reported previously in mixed connective tissue disease, in which, however, trigeminal sensory neuropathy is

frequently seen. Protected by copyright.

Involvement of the sensory portion of the trigeminal Case report occurs in various disorders. "Primary" idiopathic sensory involvement is not associated A 52 year old male, employed as a grinder in an iron with other neurological or systemic disorders, works, complained of numbness and in whereas "secondary" involvement is seen with both . He soon developed aching in his neck tumours, connective tissue diseases, demyelinating and proximal upper extremities, and numbness which disorders, trauma, or secondary to toxic substances.' started in the maxillary area on the right, but which Likewise, the causes of the carpal tunnel syndrome in one week involved the entire right side of his face. are many and varied; any condition causing an He also complained of swelling of the hands with increase of mass in the carpal tunnel can cause tightness ofthe skin, without arthralgia or Raynaud's compression of the . phenomenon. His past history was unremarkable, A recently described connective tissue disease, except for pneumoconiosis (silicosis) related to his mixed connective tissue disease, consists of over- occupational exposure to grinding dust. lapping features of progressive systemic sclerosis, General physical examination was unremarkable, http://jnnp.bmj.com/ lupus erythematosus, and polyMyositis.2 3 The except for the presence of diffuse inspiratory rales syndrome is characterised by the presence in the and swelling and induration over the fingers, and to a serum of an antibody which is specific for extractable lesser extent over the hands. Neurological examina- nuclear antigen; serum from patients with mixed tion revealed that the corneal on the right side connective tissue disease have high titre antibody to was diminished (afferent), as was pin and light touch extractable nuclear antigen and ribonucleoprotein; sensation over the entire trigeminal nerve distribution in contrast, patients with lupus erythematosus may as far as the vertex of the skull. Hypaesthesia was have normal extractable nuclear antigen titres, but present on the buccal mucosa, and on the anterior on October 1, 2021 by guest. have elevated titres of antibody to Sm antigen.2 two-thirds of the tongue on the right. Tinels sign was We report the case of a patient who presented with present at both over the median , but bilateral carpal tunnel syndromes and trigeminal there was no Phalens sign. There was no weakness or sensory neuropathy as the initial manifestations of sensory loss, and muscle stretch were all mixed connective tissue disease. present. The remainder of the neurological examina- tion was normal. Address for reprint requests: Dr FM Vincent, 211 Beaumont Place, Laboratory examinations revealed a haemoglobin Traverse City, Michigan 49684, USA. of 14-6 g/dl and a haematocrit of 44%. Erythrocyte Accepted 10 February 1980 sedimentation rate was 33 mm/hr (normal 0-20), 458 J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.43.5.458 on 1 May 1980. Downloaded from

Trigeminal sensory neuropathy and bilateral carpal tunnel syndrome 459 creatinine phosphokinase 178 IU/L (normal 30-170), finding. aldolase 16 IU (normal up to 7-5), serum glutamic Trigeminal neuropathy, usually purely sensory, oxaloacetic transaminase 69 IU/L (normal 8-30), and rarely has been reported to accompany connective lactic dehydrogenase 402 IU/L (normal 90-210). A diseases. Ashworth and Tait4 reported six urinary heavy metal and porphyrin assay were patients who developed facial numbness: three negative. Serum protein electrophoresis revealed a had systemic sclerosis, two had systemic lupus polyclonal gammopathy with a total protein of 7-5 g, erythematosus with features ofsystemic sclerosis, and albumin 4 0 g, and gamma globulin 1 9 g. Rheuma- one had polymyositis. All complained of numbness toid factor was positive at a titre of 1:40, and in the face, and was a feature in some. In three antinuclear antibody was present in a titre of 1:160 patients neuropathy was bilateral. It is possible that with a speckled pattern. Ribonucleoprotein was the patients with systemic lupus erythematosus with positive, and Sm antibodies were negative. Extract- features of systemic sclerosis would be defined today able nuclear antigen was present in a titre of as having mixed connective tissue disease. In all 1:12 500 (normal is less than 1:1000). Base views of these patients the area of involvement remained the skull and barium swallow examination were quite constant for a number of years and did not normal. A chest roentgenogram showed evidence of respond to treatment. bilateral silicosis. Bennett et a13 reviewed the neuropsychiatric Nerve conduction studies revealed the following: manifestations of mixed connective tissue disease. Of both median nerve sensory action potentials were their 11 patients, two had trigeminal neuropathy, un- absent; the right median motor conduction velocity responsive to treatment. Aseptic meningitis occurred was 56 m/s, with a distal latency of 5-7 ms; the left in four patients, convulsions in two and peripheral median motor conduction velocity was 51 m/s, with a neuropathy in two. The patients with the aseptic distal latency of 6-1 ms; conduction meningitis-like illness and one of the patients with Protected by copyright. velocities and distal latencies were normal bilaterally. convulsions responded favourably to Electromyographicexamination revealed the presence medication. These authors found a 55 % incidence of of increased insertional activity, positive sharp waves, neuropsychiatric problems in their patients with and fibrillation potentials in the proximal upper mixed connective tissue disease, but such nervous extremities, together with myopathic potentials on system involvement was not associated with any volition. A skin biopsy from the right index finger mortality, as has been the case with systemic lupus showed features consistent with . erythematosus. The patient was treated with prednisone 60 mg Trigeminal sensory neuropathy is not purely daily, and although the sensory symptoms in his confined to connective tissue diseases. Facial hands resolved, the facial numbness was unchanged. numbness may be the initial symptom of disease of the , and although it is uncommon, it Discussion is not rare.' Horowitz' reviewed 64 patients with facial numbness as an initial or presenting symptom. Mixed connective tissue disease was first described by Fifty-three patients had trigeminal involvement Sharp et a12 as an overlap syndrome of systemic secondary to other conditions: 27 had tumours ofthe

lupus erythematosus, progressive systemic sclerosis, cerebellopontine angle of base of the skull, two had http://jnnp.bmj.com/ and polymyositis. The initial impression has been intramedullary tumors, five had multiple sclerosis, that mixed connective tissue disease tends to be more six had multiple cranial nerve palsies on a viral basis, of a benign connective tissue disease, especially when three had dental or facial trauma, and two had the nervous system is involved3. The patient des- vascular diseases. In seven patients (11.-7 %) no cribed here had features consistent with systemic underlying disease was found. sclerosis (scleroderma), elevated muscle enzyme The cause of trigeminal neuropathy in mixed levels and a electromyographic examination con- connective disease is unknown. Because patients sistent with polymyositis, together with a high serum with connective tissue can develop peripheral nerve on October 1, 2021 by guest. titre of extractable nuclear antigen; thus, the dysfunction, including mixed motor-sensory neuro- diagnosis of mixed connective tissue disease fits best. pathy, sensory neuropathy or mononeuritis multiplex, Carpal tunnel syndrome occurs in many diseases a vasculitis involving the gasserian is a causing synovitis at the , and has been seen as possibility. Perhaps in mixed connective tissue the first manifestation of such diseases as lupus disease the trigeminal nerve is more selectively erythematosus and systemic sclerosis, but we have vulnerable than other parts of the nervous system. been unable to find any reports of it occurring as the Immune complex formation in the nerve itself or in initial manifestation of mixed connective tissue the sensory root could possibly cause isolated disease, although it would not be an unexpected trigeminal sensory dysfunction. Nerve biopsy from a J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.43.5.458 on 1 May 1980. Downloaded from

460 FM Vincent and R N Van Houzen patient with mixed connective tissuedisease hasshown References vasculitis, nerve connective tissue fibrosis, together with nerve infarcts and damage to both axons and 1 Horowitz SH. Isolated facial numbness: clinical myelin.6 There have been no studies significance and relation to trigeminal neuropathy. Ann Int Med 1974; 80:49-53. reported on patients with mixed connective tissue 2 Sharp GC, Irvin WS, Tan EM, Gould RG, Holman disease with trigeminal nerve dysfunction, but one HR. Mixed connective tissue disease an apparent case of a fatal transverse myelitis in a patient with distinct rheumatic disease syndrome associated with mixed connective tissue disease has been reported,6 a specific antibody to extractable nuclear antigen and the spinal cord changes were secondary to (ENA). Am J Med 1972; 52:148-59. vasculitis involving the cord, with multifocal 3 Bennett RM, Bong DM, Spargo BH. Neuropsy- necrosis seen as the end stage ofvascular involvement. chiatric problems in mixed connective tissue disease. Thus, the presence of an isolated trigeminal Am J Med 1978; 65:955-62. sensory neuropathy in a symptomatic patient should 4 Ashworth B, Tait GBW. Trigeminal neuropathy in connective tissue diseases. Neurology (Minneap) thoroughly be investigated. Because there may be a 1971; 21:609-14. long interval between the onset of symptoms and the 5 Currie DM, Bradshaw DC. in mixed discovery of the cause of the neuropathy the patient connective tissue disease presenting as progressive should be examined periodically. However, approxi- systemic sclerosis (scleroderma): case report and mately 10% of patients with isolated facial numbness literature review, abstracted. Arch Phys Med Rehabil will not develop associated neurological or systemic 1979; 60:594. diseases. One should also be aware that the carpal 6 Weiss TD, Nelson JS, Woolsey RM, Zuckner J, tunnel syndrome may rarely be the presenting Baldasarre AR. Transverse myelitis in mixed manifestation ofa connective tissue disease. connective tissue disease. Arthritis Rheum 1978; 21:982-6. Protected by copyright. We would like to thank Dr Jim Milliken for his help in evaluating our patient, and Ticia Vincent for her preparation ofthe manuscript. http://jnnp.bmj.com/ on October 1, 2021 by guest.